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Endocrine Abstracts (2016) 41 EP902 | DOI: 10.1530/endoabs.41.EP902

Endocrinology, Diabetes and Metabolism Department, Coimbra University and Hospital Center, Coimbra, Portugal.


Introduction: Craniopharyngiomas are rare epithelial tumors of the sellar and parasellar region, with high survival rates but with frequent tumor recurrence or persistence.

Methods: Information collection from clinical records and review of the epidemiology, diagnosis, treatment and follow-up of patients with diagnosis of craniopharyngioma followed in an Endocrinology Department between 1980 and 2015. Statistical analysis using SPSS v. 22.0.

Results: Forty patients were included, 50% were male. Median age by diagnosis was 36 years (minimum 6, maximum 70). In respect to clinical manifestations at diagnosis, visual alterations were present in 77.5% (n=31) and headache in 72.5% (n=29). The majority of the tumors had sellar and suprasellar involvement (77.5%); cystic component was detected in 89.7% and calcifications in 47.5%. Initial surgical approach was transcranial in 74.4%, transsphenoidal in 25.6%. From the available neuropathology results (n=29), 79.3% were adamantinomatous and 20.7% papillary. The average number of surgical interventions per patient was 2.03 (minimum 1, maximum 7). Eight patients (20.0%) were treated with radiotherapy. In the last neuroimaging evaluation, 47.5% of patients had residual tumor. Panhypopituitarism was detected in 6/30 patients preoperatively and in 23/39 in follow-up. All the isolated anterior pituitary deficits were also more frequent in follow-up. Weight gain was seen in 83.9% of patients. Prevalence of diabetes Mellitus, arterial hypertension and dyslipidemia also increased. Average follow-up time was 15.5 (±10) years. Six patients died, of whom four had been diagnosed between 6 and 14 years of age.

Conclusion: Despite the evolution in neurosurgical technique and the possibility of radiotherapy, craniopharyngiomas are still associated with high rates of tumor persistence and substantial morbidity. Optimization of the treatment of any endocrine and metabolic sequelae of the disease is essential in the management of these patients.

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